Forme fruste of isolated right ventricular endomyocardial fibrosis: A case report

Introduction. Endomyocardial fibrosis is a neglected tropical disease of unknown etiology and poor prognosis. It is endemic of tropical climates where it is the most common cause of restrictive cardiomyopathy in the second and fourth decades of life. A forme fruste of the disease is thought to be present in temperate climates where the diagnosis remains exceedingly rare. Case presentation. We describe a case of isolated right ventricular endomyocardial fibrosis in a 27-year-old Caucasian man from a temperate climate who presented initially with frank hemoptysis and pulmonary thromboembolic disease. We further describe the approach utilized in the diagnosis, the surgical treatment and the outcome of the disease. Conclusions: We conclude that endomyocardial fibrosis should be included in the differential diagnosis of apical cardiomyopathies in patients from temperate climates.